II. Epidemiology
- Incidence: Up to 39 cases per 100,000
- Usual onset at 20 to 40 years
- Gender preponderance in men by 2:1 ratio
- Ethnicity
- Most commonly affects white patients
- Rarely affects black or asian patients
III. Pathophysiology
- Idiopathic condition
- Usually associated with Gluten Sensitive Enteropathy
IV. Symptoms: Precede lesion onset by 8 to 12 hours
- Intense Pruritus
- Skin burning
V. Signs
- Grouping of lesions may occur (herpetiform-like)
- Altered pigmentation at sites of healed lesions
- Polymorphous collection of lesions
- Symmetric distribution of lesions on limbs and trunk
VI. Differential Diagnosis
- Pemphigus hermetiformis
VII. Labs
- Complete Blood Count
- Histology
- Dermal Papillae with Neutrophil microabscesses
- Dermal inflammatory infiltrate
- Subepidermal vessicles
- Blisters in the lamina lucida
- Lymphohistiocytic infiltrate at dermal vessels
- Immunofluorescence
- Granular IgA deposits in Dermal Papillae tips
- Other autoimmune lab associations variably present
VIII. Complications
- Gluten Sensitive Enteropathy associated conditions
- Autoimmune Conditions
IX. Management
- Medications
- First-Line: Dapsone
- Alternative: Sulfapyradine
- Dietary Management
- Gluten-Free Diet (improvement within 6-12 months)
- Elemental Diet (improvement may be seen in weeks)
XI. Course
- Prolonged course over years
- Spontaneous remission in one third of patients